Childhood-onset Systemic Lupus Erythematosus: clinical presentation and prognosis in 31 patients.

OBJECTIVE: To determine the clinical features at onset, the disease course, and prognostic factors in children with SLE. METHODS: The medical records of 31 patients with childhood-onset SLE were reviewed. Signs and symptoms at onset and during the course of the disease were documented as well as survival and SLICC/ACR damage index. The disease course was compared to 135 consecutive adult-onset SLE patients. RESULTS: Childhood-onset SLE most frequently presented with fatigue, arthritis, fever, weight loss, and malar rash. During follow-up, the frequency of the presence of malar rash, anemia, leukocytopenia, and anti-dsDNA antibodies was significantly higher in childhood-onset than in adult-onset patients. Mean SLICC/ACR damage index was 2.6 after 4.7 years of follow-up. The presence of arthritis, anemia, and seizures at the onset of disease resulted in a 2.6 to 3.9 times higher chance of a severe disease course. CONCLUSION: Patients with childhood-onset SLE suffer from substantial morbidity. Arthritis, anemia, and seizures at onset may be indicators of poor prognosis.

Significance of lymphoblasts in cerebrospinal fluid in newly diagnosed pediatric acute lymphoblastic malignancies with bone marrow involvement: possible benefit of dexamethasone.

We analyzed retrospectively the data on 135 children treated since 1983 for acute lymphoblastic leukaemia and non-Hodgkin's lymphoma with bone marrow involvement with respect to the presence of lymphoblasts in cerebrospinal fluid (CSF) and the number of cells in CSF, both at initial diagnosis and during follow-up. Of these children 96, 11, and 28 suffered from B-progenitor, mature B cell, and T-cell malignancies respectively. In two patients initial central nervous system involvement was documented by the presence of lymphoblasts with high CSF cell counts (B+C+ patients); 19 patients had CSF lymphoblasts with normal CSF cell counts (B+C- patients); the others had no CSF blasts and normal CSF cell counts (B-C- patients). In B+C- and B-C- patients 5-year-leukaemia-free survival was 66 and 70%, respectively (i.e., not significantly different). None of the B+C- patients experienced a first relapse in the central nervous system. Differences in outcome by comparing with reports of others may be related to the use of dexamethasone instead of prednisone in almost all of our patients.